History

Findings

There is prompt concentration of the radiotracer in the liver, with persistent radiotracer activity in the cardiac blood pool at 30 minutes (Fig. 1A). The gallbladder is not visualized at any time during the examination (Fig. 1A-C). The passage of the radiotracer into the extrahepatic biliary system and bowels is not identified at any time following injection with radiotracer (Fig. 1A-C). No significant radiotracer uptake is noted in the kidneys (Fig. 1A-C). Trace levels of the radiotracer are identified in the bladder and diaper on the 24 hour scan, consistent with urinecontamination (Fig. 1C). In the setting of persistently abnormal LFTs and elevated bilirubin levels, failure to visualize the biliary system or small bowel radiotracer accumulation by 24 hours is consistent with the clinical diagnosis of biliaryatresia.

Diagnosis

Discussion

Biliaryatresia, defined as the lack of patent extrahepatic bile ducts, is the most common cause of cholestasis in infants younger than 3 months [1]. The disease is defined by inflammation and sclerosis of the intra and extrahepatic bile ducts causing ductal luminal obliteration and eventual biliarycirrhosis and obliteration of the biliary tree [1]. The clinical forms of biliaryatresia include the perinatal type (65%-85%) and the embryonic or fetal type (15-35%). The perinatal type has postnatal onset of cholestasis, no associated congenital anomalies, and the bile duct remnants are present [1]. The embryonic or fetal type has early onset of cholestasis, no bile duct remnants, and associated congenital anomalies [1].